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A Novel Heterozygous Missense Variant (c.667G>T;p.Gly223Cys) in USH1C That Interferes With Cadherin-Related 23 and Harmonin Interaction Causes Autosomal Dominant Nonsyndromic Hearing Loss

Song JS, Bahloul A, Petit C, Kim SJ, Moon IJ, Lee J, Ki CS

BACKGROUND: Pathogenic variants of USH1C, encoding a PDZ-domain-containing protein called harmonin, have been known to cause autosomal recessive syndromic or nonsyndromic hearing loss (NSHL). We identified a causative gene in...
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Cortical Deafness and Tinnitus Following Sequential Bilateral Putaminal Hemorrhage

Yang S, Chen S, Zhu J, Han X

No abstract available.
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Clinical and Genetic Spectrum of ATP1A3-Related Disorders in a Korean Pediatric Population

Kim WJ, Shim YK, Choi SA, Kim SY, Kim H, Hwang H, Choi J, Kim KJ, Chae JH, Lim BC

BACKGROUND AND PURPOSE: The aim of this study was to expand the understanding of the genotype-phenotype spectrum of ATP1A3-related disorders and to evaluate the therapeutic effect of a ketogenic diet...
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A Case of Bilateral Semicircular Canal Dysplasia with Normal Cochlear Found in Bilateral Vestibulopathy

Lee S, Choi SW

A 25-year-old woman presented with atypical dizziness with oscillopsia. The neuro-otological evaluations showed bilateral vestibulopathy with mild sensorineural hearing loss. Computed tomography and magnetic resonance imaging demonstrated bilateral isolated lateral...
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Comparison of Treatment Outcomes between Intratympanic Steroid Injection and Oral Diuretics in Patients with Acute Low Frequency Sensorineural Hearing Loss with Vertigo

An YH, Shim HJ

OBJECTIVES: Sudden sensorineural hearing loss and Meniere disease can be managed with intratympanic steroid injection (ITSI) as a primary treatment. The aim of this study was to compare the effectiveness...
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The Value of Posterior Semicircular Canal Function in Predicting Hearing Recovery of Sudden Sensorineural Hearing Loss

Shin JW, Kim SW, Kim YW, Jang W, Kim BH, Lim YS, Park SW, Cho CG, Park JH

OBJECTIVES: Postulated etiologies for idiopathic sudden sensorineural hearing loss (SSNHL) include viral cochleitis, microvascular events. If SSNHL is caused by vascular compromise of common cochlear artery that supplies cochlea and...
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Clinical and genetic characteristics of Korean patients with IARS2-related disorders

Lee JS, Kim MJ, Kim SY, Lim BC, Kim KJ, Choi M, Seong MW, Chae JH

PURPOSE: Genetic defects in the nuclear-encoded mitochondrial aminoacyl-tRNA synthetases were first identified as causes of various disorders in 2007. Variants in IARS2, which encodes a mitochondrial isoleucyl-tRNA synthetase, were first...
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Tumor Growth and Hearing Changes in the Natural Progress of Vestibular Schwannoma

Lee SJ, Lee YJ, Kim BG, Lee CK, Lee BD, Lee JD

BACKGROUND AND OBJECTIVES: Vestibular schwannoma is a benign neoplasm originating from the vestibular nerve, and the most common symptom caused by the tumor is unilateral hearing loss. This study is...
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Translation and Linguistic Validation of Korean Version of Musical Background Questionnaire

An YH, Choi BY, Kim BJ, Choi JW, Park MK, Han GC, Cheon BC, Shim HJ, Chai MS, Gfeller K, Ahn J, Moon IJ, Cho YS

BACKGROUND AND OBJECTIVES: The Musical Background Questionnaire (MBQ) has been developed to assess formal musical training and listening enjoyment. The aims of this study were to translate MBQ into Korean...
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Comparison of the Results between the First Steroid Treatment Group and the Retreatment Group with Idiopathic Sudden Sensorineural Hearing Loss

Ko JB, Kim CH, Jo KH, Lee HH, Kwon JH

BACKGROUND AND OBJECTIVES: Sudden sensoryneural hearing loss (SSNHL) is an otological emergency disease of a non-specific cause. If there is no improvement or if the degree of hearing loss is...
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Ocular Manifestations, Treatment Outcome and Clinical Course of Infective Keratitis of Keratitis-Ichthyosis-Deafness Syndrome

Son KY, Paik DW, Cho EH, Shin D, Woo HI, Chung TY, Lim DH

PURPOSE: To report the ocular manifestations and treatment outcomes of infective keratitis in a patient with keratitis-ichthyosis-deafness (KID) syndrome and to report a newly discovered mutation in the GJB2 gene...
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Two Cases of Localized Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Manifested with Intractable Otitis Media

Lee E, Chung WH

Localized form of myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis (MPO-AAV) in the ear is a rare disease. It manifests as a sudden otalgia with hearing loss accompanied by dizziness and facial nerve...
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Safety and efficacy of transcutaneous bone conduction implant surgery for hearing improvement in microtia patients with bilateral hearing impairment

Cheon JH, Lee HC, Im GJ, Park JY, Park C

BACKGROUND: In microtia patients with bilateral hearing impairment, hearing improvement is crucial for language development and performance. External auditory canal reconstruction (EACR) has been performed to improve hearing, but often...
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Response of Glucocorticoid Receptor Alpha and Histone Deacetylase 2 to Glucocorticoid Treatment Predicts the Prognosis of Sudden Sensorineural Hearing Loss

Zhang X, Chen J, Gao Z, Qi H, Dai Y, She W

OBJECTIVES: To investigate glucocorticoid receptor (GR) and histone deacetylase 2 (HDAC2) gene expression and protein levels in peripheral blood mononuclear cells (PBMCs) of patients with severe or profound sudden sensorineural...
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Speech Perception and Gap Detection Performance of Single-Sided Deafness under Noisy Conditions

Kwak C, Kim S, Lee J, Seo Y, Kong T, Han W

BACKGROUND AND OBJECTIVES: Many studies have reported no benefit of sound localization, but improved speech understanding in noise after treating patients with single-sided deafness (SSD). Furthermore, their performances provided a...
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Genetics of Hearing Loss in North Iran Population: An Update of Spectrum and Frequency of GJB2 Mutations

Koohiyan M, Azadegan-Dehkordi F, Koohian F, Hashemzadeh-Chaleshtori M

Diagnosis of pre-lingual hearing loss (HL) is difficult owing to the high number of genes responsible. The most frequent cause of HL is DFNB1 due to mutations in the GJB2...
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A Case of Rapid Progressive Neurosyphilis in Patient with Ankylosing Spondylitis Who Is Treating Anti-interleukin 17A Monoclonal Antibody, Secukinumab

Lee SJ, Park HK, Kim YS

Anti-interleukin 17A agent, secukinumab is remarkably effective for treating patients with ankylosing spondylitis. However, the main safety concern of secukinumab is an increased risk of infection. Generally, neurosyphilis occurs a...
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The Influence of Education Level on Tinnitus and Quality of Life in Korean Adults

Jung DJ, Yoo MH, Jung YJ, Lee HJ, Lee KY

BACKGROUND AND OBJECTIVES: We aimed to evaluate the effect of the level of education on the prevalence of tinnitus and quality of life (QoL) in the Korean population. SUBJECTS AND METHOD:...
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Two Cases of Pediatric Pneumolabyrinth with Traumatic Tympanic Membrane Perforation after Penetrating Injury

You YS, Koh JH, Kim BJ, Lee EJ

Pneumolabyrinth is an uncommon condition in which air is present in the vestibule or cochlear. It is rarely found, even in otic capsule violating fractures or in transverse fracture of...
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Applications of photobiomodulation in hearing research: from bench to clinic

Lee JH, Kim S, Jung JY, Lee MY

Hearing loss is very common and economically burdensome. No accepted therapeutic modality for sensorineural hearing loss is yet available; most clinicians emphasize rehabilitation, placing hearing aids and cochlear implants. Photobiomodulation...
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